Highly malignant disease in childhood-onset arrhythmogenic right ventricular cardiomyopathy.

Marit Kristine Smedsrud,Monica Chivulescu,Marianne Inngjerdingen Forså,Isotta Castrini,Eivind Westrum Aabel,Christine Rootwelt-Norberg,Martin Prøven Bogsrud,Thor Edvardsen, Nina Eide Hasselberg,Andreas Früh,Kristina Hermann Haugaa

European heart journal（2022）

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摘要

In a paediatric ARVC cohort, there was a high incidence of severe cardiac events and half of them occurred in children ≤12 years of age. The ARVC penetrance in genotype positive paediatric relatives was 18%. These findings of a high-malignant phenotype in childhood-onset ARVC indicate a need for ARVC family screening at younger age than currently recommended.

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关键词

Arrhythmogenic right ventricular cardiomyopathy,Children,Disease penetrance,Family screening,Heart transplantation,Ventricular arrhythmia

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